Image Description
Papillary fibroelastoma is a small papillary lesion with sea-anemone-like gross appearance that is usually found on the surface of the cardiac valves or other endocardial surfaces. It is not certain whether they are true neoplasms or reactive lesions. They are usually discovered incidentally at autopsy, cardiac surgery, or on high-resolution imaging studies.
Microscopically, it is composed of numerous branching papillary fronds arising from a common stalk. The individual fronds are composed of an avascular core of hyalinized hypocellular stroma with elastic fibers. The fronds are lined by one or more layers of endocardial cells.
Image courtesy of: Ankur Sangoi, MD; @slusagar; used with permission.
About the Disease
Primary cardiac tumors are extremely uncommon. Secondary tumors (metastases to heart) are 20-50 times more common than primary cardiac tumors and yet still relatively rare. Of primary cardiac tumors, almost 75% are benign and the remaining 25% are malignant.Benign Primary Cardiac Tumors: Myxoma (50-70% of cases), rhabdomyoma, lipoma and lipomatous hypertrophy of interatrial septum, papillary fibroelastoma, and fibroma. Angiomas and teratomas are found mainly in children. Malignant Primary Cardiac Tumors: Mostly sarcomas, including angiosarcoma (30%), rhabdomyosarcoma (20%), leiomyosarcoma, liposarcoma, osteosarcoma, fibrosarcoma, and undifferentiated pleomorphic sarcoma. Other tumors include mesothelioma and lymphoma.Secondary cardiac tumors are far more frequent than primary cardiac tumors. The most common primary sites include melanoma, lung, breast, kidney, and lymphomas. Pericardium is usually affected, resulting in pericardial effusion.Cardiac tumors may be asymptomatic and found incidentally or present with obstructive, embolic, arrhythmic or systemic symptoms, depending upon the size and location of the mass. References: 1. Miller, D. V. & Revelo M. P. (2023). Diagnostic Pathology - Cardiovascular. 3rd Edition, Elsevier.2. Sheppard, M. N. (2022). Practical Cardiovascular Pathology 3rd Edition, CRC Press.3. Maximilian B. & Butany J. (2022). Cardiovascular Pathology 5th Edition, Academic Press.